Understanding and Managing Moyamoya Disease (Podcast)

Moyamoya disease is a rare cerebrovascular disorder characterized by progressive narrowing of the large intracranial arteries. While it is often seen in patients with large-artery intracranial occlusive disease, etiologies are numerous and the cause is often unknown. As a result, misdiagnosis and delayed treatment are common.

“The approach often is, ‘This patient has a blood vessel problem, and we need to revascularize,’” notes Andrew Russman, DO, Medical Director of the Comprehensive Stroke Program at Cleveland Clinic. “We need to take a step back. For the majority of patients, we have at least some time to investigate the underlying etiologies. Taking a comprehensive approach is critically important to long-term management for those who have moyamoya disease or vasculopathy.”

In the latest episode of Cleveland Clinic’s Neuro Pathways podcast, Dr. Russman discusses the management and treatment of moyamoya disease, including:

• The characteristics and mechanisms associated with the disease
• The difference between moyamoya disease and moyamoya syndrome
• Common clinical presentations, including ischemic symptoms and intraparenchymal hemorrhage
• A detailed review of the diagnostic workup
• Surgical and medical treatment options
• Ongoing research into this cerebrovascular disorder

Click the podcast player above to listen to the 30-minute episode now, or read on for a short edited excerpt. Check out more Neuro Pathways episodes at clevelandclinic.org/neuropodcast or wherever you get your podcasts.

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Excerpt from the podcast

Podcast host Glen Stevens, DO, PhD: What can you tell us about ongoing research that may shed further light on these conditions?

Dr. Russman: There aren’t a lot of randomized studies in populations of patients with moyamoya. I think that’s because many treating clinicians don’t have clinical equipoise for not performing revascularization on somebody who presents with ischemia and a very clear moyamoya vasculopathy. However, we have a lot of uncertainties about the approach to cases that are likely to be moyamoya syndrome, where we see such a heterogeneous group of patients and different conditions that are associated with the vasculopathies.

I think some of the important work that has to be done over time is to characterize this population more appropriately in terms of applying a standard evaluation and then understanding what are the propensities or likelihoods of detecting this within a large center, such as Cleveland Clinic. That is an effort that’s underway on our part. Others have looked at this and found variable percentages of these presentations of the subtypes.

From a treatment perspective, it still comes down to whether the patient is having recurrent ischemia and whether they are unable to maintain adequate flow, in which case revascularization is critically important to preventing future events. Whether it’s moyamoya disease or the syndrome of moyamoya, revascularization or surgical revascularization is the mainstay of therapy. And we would like to better understand how to combine medical treatments with surgical treatments, especially for patients with moyamoya syndrome.